Extramammary Paget's disease of the perineum: Avoiding pitfalls in diagnosis and management

Abstract

Extramammary Paget's disease (EMPD) is a rare entity, especially in the perinoscrotal region, and typically presents in elderly white patients as a pruritic white or red patch in the area of distribution of apocrine glands. Typically, it affects a single site. Since its manifestations are insidious and easily misdiagnosed, the appropriate management is delayed. Management of this problem is complex and effective treatment can not only lower recurrence rates but also provide an optimal reconstructive result. The present report describes three patients with scrotal EMPD. Based on literature search, the etiopathology, diagnosis and management of these lesions is discussed. Reconstructive options, with special emphasis on scrotal lesions, are also discussed.

Keywords: Cutaneous pruritic patch; Extramammary Paget’s disease; Scrotal lesion.

Figure 1)

Preoperative view of patient in Case 1 reveals an erythematous area along the medial aspect of the thigh and extending to the scrotum on the right side. Note the lack of ulceration and a seemingly well-defined macroscopic border

Figure 2)

Postoperative view following complete resection of the lesion followed by reconstruction of the scrotal defect using a fasciocutaneous advancement flap from the scrotum itself and a rhomboid flap from the medial aspect of the thigh to close the defect in that area

Figure 3)

Low power microscopic view of the specimen reveals tumour cells infiltrating the skin and the adnexa

Figure 4)

High power microscopy confirm the presence of Paget cells – abundant and large, these cells are characteristic with pale cytoplasm.Distributed singly and in groups they are noted throughout the epidermis and the epithelia of the adnexa. Upper regions of the dermis show dense inflammatory cells